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Pharmacoperones as Novel Therapeutics for Diverse Protein Conformational Diseases
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Type I procollagen: The gene‐protein system that harbors most of the mutations causing osteogenesis imperfecta and probably more common heritable disorders of connective tissue
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Ubiquitin at Fox Chase (Nobel Lecture)
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Intracellular Protein Degradation: From a Vague Idea, through the Lysosome and the Ubiquitin–Proteasome System, and onto Human Diseases and Drug Targeting (Nobel Lecture)
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The Ubiquitin System for Protein Degradation and Some of Its Roles in the Control of the Cell‐Division Cycle (Nobel Lecture)
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A Brief History of G‐Protein Coupled Receptors (Nobel Lecture)
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Molecular chaperones in cellular protein folding
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Correctors of Protein Trafficking Defects Identified by a Novel High‐Throughput Screening Assay
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The molecular basis for the recognition of misfolded glycoproteins by the UDP‐Glc:glycoprotein glucosyltransferase.
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Characterization of a Mutant α-Galactosidase Gene Product for the Late-Onset Cardiac Form of Fabry Disease
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Galactose Stabilizes Various Missense Mutants of α-Galactosidase in Fabry Disease
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Morphological Analysis of the Transfer of VSV ts-045 G Glycoprotein from the Endoplasmic Reticulum to the Intermediate Compartment in Vero Cells
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Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate
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Quantitative correlation between the residual activity of β-hexosaminidase A and arylsulfatase A and the severity of the resulting lysosomal storage disease
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The pharmacological chaperone 1‐deoxygalactonojirimycin increases α‐galactosidase A levels in Fabry patient cell lines
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A GCase Chaperone Improves Motor Function in a Mouse Model of Synucleinopathy
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Human α1-proteinase inhibitor
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The LDL receptor locus in familial hypercholesterolemia: Multiple mutations disrupt transport and processing of a membrane receptor
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Conversion of a secretory protein into a transmembrane protein results in its transport to the golgi complex but not to the cell surface
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An hsp70-like protein in the ER: Identity with the 78 kd glucose-regulated protein and immunoglobulin heavy chain binding protein
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Heterogeneous mutations in the β subunit common to the LFA-1, Mac-1, and p150,95 glycoproteins cause leukocyte adhesion deficiency
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Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
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Identification of a protein required for disulfide bond formation in vivo
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Heat shock, stress proteins, chaperones, and proteotoxicity
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Degradation of CFTR by the ubiquitin-proteasome pathway
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Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
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Glucose trimming and reglucosylation determine glycoprotein association with calnexin in the endoplasmic reticulum
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UPRmt-mediated cytoprotection and organismal aging
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Functional characterization of novel melanocortin-3 receptor mutations identified from obese subjects
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Pharmacological chaperones for the misfolded melanocortin-4 receptor associated with human obesity
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Ubiquitin–Proteasome System and mitochondria — Reciprocity
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Regulation of proteasome activity in health and disease
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Pathologic endoplasmic reticulum stress induced by glucotoxic insults inhibits adipocyte differentiation and induces an inflammatory phenotype
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Protein disulfide isomerase
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Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs
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Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease
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PRIMA-1 Reactivates Mutant p53 by Covalent Binding to the Core Domain
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Disulfide bonds in ER protein folding and homeostasis
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Mistranslation-Induced Protein Misfolding as a Dominant Constraint on Coding-Sequence Evolution
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Blinded by the Light: The Growing Complexity of p53
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Gaucher Disease Glucocerebrosidase and α-Synuclein Form a Bidirectional Pathogenic Loop in Synucleinopathies
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Regulation of Organismal Proteostasis by Transcellular Chaperone Signaling
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XBP-1 Is a Cell-Nonautonomous Regulator of Stress Resistance and Longevity
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Inefficient SRP Interaction with a Nascent Chain Triggers a mRNA Quality Control Pathway
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Lipid Biosynthesis Coordinates a Mitochondrial-to-Cytosolic Stress Response
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A Chaperome Subnetwork Safeguards Proteostasis in Aging and Neurodegenerative Disease
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Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR
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Pathogenesis of Human Mitochondrial Diseases Is Modulated by Reduced Activity of the Ubiquitin/Proteasome System
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Protein disulfide isomerases in neurodegeneration: From disease mechanisms to biomedical applications
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Mutant p53 reactivation by small molecules makes its way to the clinic
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Designer protein disaggregases to counter neurodegenerative disease
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A single nucleotide polymorphism in the CCR3 gene ablates receptor export to the plasma membrane
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Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking
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Glycosylation Quality Control by the Golgi Structure
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The molecular chaperone Hsp104—A molecular machine for protein disaggregation
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Molecular mechanisms of the neural melanocortin receptor dysfunction in severe early onset obesity
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Refolding of misfolded mutant GPCR: Post-translational pharmacoperone action in vitro
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Increased plasma membrane expression of human follicle-stimulating hormone receptor by a small molecule thienopyr(im)idine
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Receptor antagonism/agonism can be uncoupled from pharmacoperone activity
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Degradation of Misfolded Proteins Prevents ER-Derived Oxidative Stress and Cell Death
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Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation
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Autophagy and the Integrated Stress Response
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The Heat Shock Response: Life on the Verge of Death
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CFTR: folding, misfolding and correcting the ΔF508 conformational defect
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The Roles of PINK1, Parkin, and Mitochondrial Fidelity in Parkinson’s Disease
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Inactivating mutations of G protein-coupled receptors and diseases: Structure-function insights and therapeutic implications
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Constitutive activation of G protein-coupled receptors and diseases: Insights into mechanisms of activation and therapeutics
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“Pharmacoperone”: What's in a word?
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Transitioning pharmacoperones to therapeutic use: In vivo proof-of-principle and design of high throughput screens
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Golgi linked protein glycosylation and associated diseases
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N-linked sugar-regulated protein folding and quality control in the ER
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A trip to the ER: coping with stress
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Retrograde transport on the COG railway
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Proteostasis impairment in protein-misfolding and -aggregation diseases
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New Insights into the Physiological Role of Endoplasmic Reticulum-Associated Degradation
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With the Help of MOM: Mitochondrial Contributions to Cellular Quality Control
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Pharmacological chaperones: potential treatment for conformational diseases
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Flagging and docking: dual roles for N-glycans in protein quality control and cellular proteostasis
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Cytosolic Proteostasis Networks of the Mitochondrial Stress Response
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Novel pharmacological strategies to treat cystic fibrosis
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Mitochondrial quality control in the myocardium: Cooperation between protein degradation and mitophagy
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The stabilization of proteins by osmolytes
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Biosynthesis, processing, and secretion of M and Z variant human alpha 1-antitrypsin.
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Transport of secretory and membrane glycoproteins from the rough endoplasmic reticulum to the Golgi. A rate-limiting step in protein maturation and secretion.
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Acceleration of Reactivation of Reduced Bovine Pancreatic Ribonuclease by a Microsomal System from Rat Liver
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The Lebanese allele at the low density lipoprotein receptor locus. Nonsense mutation produces truncated receptor that is retained in endoplasmic reticulum.
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Hsp104, Hsp70, and Hsp40
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ER Quality Control: The Cytoplasmic Connection
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A contradictory treatment for lysosomal storage disorders: inhibitors enhance mutant enzyme activity
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On the frequency of protein glycosylation, as deduced from analysis of the SWISS-PROT database
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ER quality control: towards an understanding at the molecular level
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Lectins as chaperones in glycoprotein folding
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Aggresomes, inclusion bodies and protein aggregation
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Chapter 6 Mutations in Melanocortin‐4 Receptor and Human Obesity
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Chemical and pharmacological chaperones as new therapeutic agents
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Native State Kinetic Stabilization as a Strategy To Ameliorate Protein Misfolding Diseases: A Focus on the Transthyretin Amyloidoses
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Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures
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Thermodynamic and kinetic examination of protein stabilization by glycerol
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Rescue of ΔF508 and Other Misprocessed CFTR Mutants by a Novel Quinazoline Compound
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Pharmacoproteomics of 4-Phenylbutyrate-Treated IB3-1 Cystic Fibrosis Bronchial Epithelial Cells
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Correlation between enzyme activity and substrate storage in a cell culture model system for Gaucher disease
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Traffic Jam: A Compendium of Human Diseases that Affect Intracellular Transport Processes
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Secretory Pathway Quality Control Operating in Golgi, Plasmalemmal, and Endosomal Systems
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Traffic Jams II: An Update of Diseases of Intracellular Transport
inferredcites →https://doi.org/10.1038/33416openalex-snapshot
Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism
inferredcites →https://doi.org/10.1038/35008096openalex-snapshot
Rapid degradation of a large fraction of newly synthesized proteins by proteasomes
inferredcites →https://doi.org/10.1038/35050509openalex-snapshot
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
inferredcites →https://doi.org/10.1038/35057149openalex-snapshot
A map of human genome sequence variation containing 1.42 million single nucleotide polymorphisms
inferredcites →https://doi.org/10.1038/358761a0openalex-snapshot
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
inferredcites →https://doi.org/10.1038/36285openalex-snapshot
Mutation of the mouse klotho gene leads to a syndrome resembling ageing.
inferredcites →https://doi.org/10.1038/372475a0openalex-snapshot
Protein disaggregation mediated by heat-shock protein Hspl04
inferredcites →https://doi.org/10.1038/381571a0openalex-snapshot
Molecular chaperones in cellular protein folding
inferredcites →https://doi.org/10.1038/42276openalex-snapshot
Mutant analysis links the translocon and BiP to retrograde protein transport for ER degradation
inferredcites →https://doi.org/10.1038/47062openalex-snapshot
Glycoproteins form mixed disulphides with oxidoreductases during folding in living cells
inferredcites →https://doi.org/10.1038/4801openalex-snapshot
Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor
inferredcites →https://doi.org/10.1038/cr.2013.169openalex-snapshot
Historical landmarks of autophagy research
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The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease
inferredcites →https://doi.org/10.1038/mt.2009.53openalex-snapshot
The Pharmacological Chaperone N-butyldeoxynojirimycin Enhances Enzyme Replacement Therapy in Pompe Disease Fibroblasts
inferredcites →https://doi.org/10.1038/nature02046openalex-snapshot
Global analysis of protein expression in yeast
inferredcites →https://doi.org/10.1038/nature02262openalex-snapshot
Quality control in the endoplasmic reticulum protein factory
inferredcites →https://doi.org/10.1038/nature02263openalex-snapshot
Protein degradation and protection against misfolded or damaged proteins
inferredcites →https://doi.org/10.1038/nature02265openalex-snapshot
Therapeutic approaches to protein-misfolding diseases
inferredcites →https://doi.org/10.1038/nature09299openalex-snapshot
Enhancement of proteasome activity by a small-molecule inhibitor of USP14
inferredcites →https://doi.org/10.1038/nature10317openalex-snapshot
Molecular chaperones in protein folding and proteostasis
inferredcites →https://doi.org/10.1038/nature14859openalex-snapshot
A cytosolic network suppressing mitochondria-mediated proteostatic stress and cell death
inferredcites →https://doi.org/10.1038/nature14951openalex-snapshot
Mistargeted mitochondrial proteins activate a proteostatic response in the cytosol
inferredcites →https://doi.org/10.1038/nature17989openalex-snapshot
Maintenance and propagation of a deleterious mitochondrial genome by the mitochondrial unfolded protein response
inferredcites →https://doi.org/10.1038/ncb0805-766openalex-snapshot
ERAD: the long road to destruction
inferredcites →https://doi.org/10.1038/nchembio.1770openalex-snapshot
Pharmacological folding chaperones act as allosteric ligands of Frizzled4
inferredcites →https://doi.org/10.1038/nchembio850openalex-snapshot
Structure of acid β-glucosidase with pharmacological chaperone provides insight into Gaucher disease
inferredcites →https://doi.org/10.1038/ng.439openalex-snapshot
Genome-wide association study identifies variants at CLU and CR1 associated with Alzheimer's disease
inferredcites →https://doi.org/10.1038/ng.440openalex-snapshot
Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease
inferredcites →https://doi.org/10.1038/ng0193-7openalex-snapshot
Isolation of a candidate gene for Menkes disease and evidence that it encodes a copper–transporting ATPase
inferredcites →https://doi.org/10.1038/ng0992-21openalex-snapshot
Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis
inferredcites →https://doi.org/10.1038/nm0302-282openalex-snapshot
Restoration of the tumor suppressor function to mutant p53 by a low-molecular-weight compound
inferredcites →https://doi.org/10.1038/nm1066openalex-snapshot
Protein aggregation and neurodegenerative disease
inferredcites →https://doi.org/10.1038/nrm.2016.23openalex-snapshot
Mitonuclear communication in homeostasis and stress
inferredcites →https://doi.org/10.1038/nrm1052openalex-snapshot
Quality control in the endoplasmic reticulum
inferredcites →https://doi.org/10.1038/nrm2199openalex-snapshot
Signal integration in the endoplasmic reticulum unfolded protein response
inferredcites →https://doi.org/10.1038/nrm2993openalex-snapshot
Cellular strategies for controlling protein aggregation
inferredcites →https://doi.org/10.1038/nrm3984openalex-snapshot
New roles for mitochondrial proteases in health, ageing and disease
inferredcites →https://doi.org/10.1038/nrn1007openalex-snapshot
Unfolding the role of protein misfolding in neurodegenerative diseases
inferredcites →https://doi.org/10.1038/nrneurol.2010.180openalex-snapshot
ATP7A-related copper transport diseases—emerging concepts and future trends
inferredcites →https://doi.org/10.1038/nsb0595-363openalex-snapshot
The Z type variation of human α1-antitrypsin causes a protein folding defect
inferredcites →https://doi.org/10.1038/nsmb.2971openalex-snapshot
A molecular chaperone breaks the catalytic cycle that generates toxic Aβ oligomers
inferredcites →https://doi.org/10.1038/onc.2009.425openalex-snapshot
Mutant p53 reactivation by PRIMA-1MET induces multiple signaling pathways converging on apoptosis
inferredcites →https://doi.org/10.1038/sj.bjp.0707490openalex-snapshot
A day in the life of a G protein‐coupled receptor: the contribution to function of G protein‐coupled receptor dimerization
inferredcites →https://doi.org/10.1038/sj.emboj.7601930openalex-snapshot
The two faces of protein misfolding: gain‐ and loss‐of‐function in neurodegenerative diseases
inferredcites →https://doi.org/10.1038/sj.onc.1203434openalex-snapshot
Quantitative analysis of residual folding and DNA binding in mutant p53 core domain: definition of mutant states for rescue in cancer therapy
inferredcites →https://doi.org/10.1038/sj.onc.1205362openalex-snapshot
Characterization of the p53-rescue drug CP-31398 in vitro and in living cells
inferredcites →https://doi.org/10.1038/sj.onc.1208419openalex-snapshot
PRIMA-1MET synergizes with cisplatin to induce tumor cell apoptosis
inferredcites →https://doi.org/10.1038/sj.onc.1210291openalex-snapshot
Structure–function–rescue: the diverse nature of common p53 cancer mutants
inferredcites →https://doi.org/10.1042/bj20070479openalex-snapshot
Mutant α-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin
inferredcites →https://doi.org/10.1056/nejm197501232920403openalex-snapshot
Purification and Partial Characterization of PAS-Positive Inclusion Bodies from the Liver in Alpha1-Antitrypsin Deficiency
inferredcites →https://doi.org/10.1056/nejm197509182931203openalex-snapshot
Characterization of α1-Antitrypsin in the Inclusion Bodies from the Liver in α1-Antitrypsin Deficiency
inferredcites →https://doi.org/10.1056/nejm198702193160804openalex-snapshot
A Study of the Molecular Pathology of Sucrase-Isomaltase Deficiency
inferredcites →https://doi.org/10.1056/nejm200107053450104openalex-snapshot
Improvement in Cardiac Function in the Cardiac Variant of Fabry's Disease with Galactose-Infusion Therapy
inferredcites →https://doi.org/10.1056/nejmoa1510198openalex-snapshot
Treatment of Fabry’s Disease with the Pharmacologic Chaperone Migalastat
inferredcites →https://doi.org/10.1073/pnas.051499698openalex-snapshot
Defective trafficking and function of K ATP channels caused by a sulfonylurea receptor 1 mutation associated with persistent hyperinsulinemic hypoglycemia of infancy
inferredcites →https://doi.org/10.1073/pnas.0605928103openalex-snapshot
The iminosugar isofagomine increases the activity of N370S mutant acid β-glucosidase in Gaucher fibroblasts by several mechanisms
inferredcites →https://doi.org/10.1073/pnas.0708725104openalex-snapshot
Signal sequence mutation in autosomal dominant form of hypoparathyroidism induces apoptosis that is corrected by a chemical chaperone
inferredcites →https://doi.org/10.1073/pnas.0805326105openalex-snapshot
Targeted rescue of a destabilized mutant of p53 by an in silico screened drug
inferredcites →https://doi.org/10.1073/pnas.0902882106openalex-snapshot
Collapse of proteostasis represents an early molecular event in Caenorhabditis elegans aging
inferredcites →https://doi.org/10.1073/pnas.1015723108openalex-snapshot
Rescue of expression and signaling of human luteinizing hormone G protein-coupled receptor mutants with an allosterically binding small-molecule agonist
inferredcites →https://doi.org/10.1073/pnas.1105787108openalex-snapshot
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
inferredcites →https://doi.org/10.1073/pnas.1203924109openalex-snapshot
Pharmacological chaperones for human α- N -acetylgalactosaminidase
inferredcites →https://doi.org/10.1073/pnas.1315194110openalex-snapshot
Restoration of testis function in hypogonadotropic hypogonadal mice harboring a misfolded GnRHR mutant by pharmacoperone drug therapy
inferredcites →https://doi.org/10.1073/pnas.192582899openalex-snapshot
Chemical chaperones increase the cellular activity of N370S β-glucosidase: A therapeutic strategy for Gaucher disease
inferredcites →https://doi.org/10.1073/pnas.220417997openalex-snapshot
Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis
inferredcites →https://doi.org/10.1073/pnas.241629998openalex-snapshot
A peptide that binds and stabilizes p53 core domain: Chaperone strategy for rescue of oncogenic mutants
inferredcites →https://doi.org/10.1073/pnas.2536657100openalex-snapshot
Chemical chaperone therapy for brain pathology in G M1 -gangliosidosis
inferredcites →https://doi.org/10.1073/pnas.82.13.4423openalex-snapshot
Transport to cell surface of intestinal sucrase-isomaltase is blocked in the Golgi apparatus in a patient with congenital sucrase-isomaltase deficiency.
inferredcites →https://doi.org/10.1073/pnas.82.20.6918openalex-snapshot
The cellular defect in alpha 1-proteinase inhibitor (alpha 1-PI) deficiency is expressed in human monocytes and in Xenopus oocytes injected with human liver mRNA.
inferredcites →https://doi.org/10.1073/pnas.87.13.4991openalex-snapshot
Role of the intradiscal domain in rhodopsin assembly and function.
inferredcites →https://doi.org/10.1073/pnas.90.5.1977openalex-snapshot
Apolipoprotein E: high-avidity binding to beta-amyloid and increased frequency of type 4 allele in late-onset familial Alzheimer disease.
inferredcites →https://doi.org/10.1073/pnas.91.19.9014openalex-snapshot
A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.
inferredcites →https://doi.org/10.1073/pnas.91.3.913openalex-snapshot
Role of N-linked oligosaccharide recognition, glucose trimming, and calnexin in glycoprotein folding and quality control.
inferredcites →https://doi.org/10.1073/pnas.94.12.6210openalex-snapshot
Aberrant retention of tyrosinase in the endoplasmic reticulum mediates accelerated degradation of the enzyme and contributes to the dedifferentiated phenotype of amelanotic melanoma cells
inferredcites →https://doi.org/10.1073/pnas.94.26.14338openalex-snapshot
Thermodynamic stability of wild-type and mutant p53 core domain
inferredcites →https://doi.org/10.1073/pnas.97.11.5889openalex-snapshot
Endoplasmic reticulum retention is a common defect associated with tyrosinase-negative albinism
inferredcites →https://doi.org/10.1073/pnas.97.4.1796openalex-snapshot
Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency
inferredcites →https://doi.org/10.1074/jbc.270.50.29819openalex-snapshot
Differential Glycosylation and Intracellular Trafficking for the Long and Short Isoforms of the D2 Dopamine Receptor
inferredcites →https://doi.org/10.1074/jbc.270.9.4697openalex-snapshot
The Molecular Chaperone Calnexin Binds Glc1Man9GlcNAc2 Oligosaccharide as an Initial Step in Recognizing Unfolded Glycoproteins
inferredcites →https://doi.org/10.1074/jbc.271.2.635openalex-snapshot
Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis Mutation
inferredcites →https://doi.org/10.1074/jbc.271.37.22791openalex-snapshot
Degradation of a Mutant Secretory Protein, α1-Antitrypsin Z, in the Endoplasmic Reticulum Requires Proteasome Activity
inferredcites →https://doi.org/10.1074/jbc.271.4.2139openalex-snapshot
The Mutation Gly142→ Glu in Human Lipoprotein Lipase Produces a Missorted Protein That Is Diverted to Lysosomes
inferredcites →https://doi.org/10.1074/jbc.274.11.6875openalex-snapshot
Clusterin Has Chaperone-like Activity Similar to That of Small Heat Shock Proteins
inferredcites →https://doi.org/10.1074/jbc.274.13.9029openalex-snapshot
Mutant Vasopressin Precursors That Cause Autosomal Dominant Neurohypophyseal Diabetes Insipidus Retain Dimerization and Impair the Secretion of Wild-type Proteins
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What points at this work
Incoming claims: where this work appears in other papers’ reference lists and arguments.
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Intracellular Trafficking of G Protein-Coupled Receptors to the Cell Surface Plasma Membrane in Health and Disease
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Therapeutic strategies for diseases caused by loss-of-function mutations in G protein-coupled receptors
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Melanocortin-1 receptor mutations and pigmentation: Insights from large animals
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Mutations in melanocortin-4 receptor: From fish to men
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Lysosomal functions and dysfunctions: Molecular and cellular mechanisms underlying Gaucher disease and its association with Parkinson disease
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Ligand steric effects of nano-inhibitors on Aβ fibrillation at the nano-bio interfaces
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trans, trans-2-C-Aryl-3,4-dihydroxypyrrolidines as potent and selective β-glucosidase inhibitors: Pharmacological chaperones for Gaucher disease
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Rab43 GTPase directs postsynaptic trafficking and neuron-specific sorting of G protein–coupled receptors
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Molecular chaperones and G protein-coupled receptor maturation and pharmacology
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Panoramic characterization of proposed ligands for GPR3: oleoylethanolamide as a pharmacological chaperone
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Naturally occurring mutations in G protein-coupled receptors associated with obesity and type 2 diabetes mellitus
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Structural mechanism for tyrosine hydroxylase inhibition by dopamine and reactivation by Ser40 phosphorylation
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Concise asymmetric synthesis of new enantiomeric C -alkyl pyrrolidines acting as pharmacological chaperones against Gaucher disease
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Turning the structure of the Aβ 42 peptide by different functionalized carbon nanotubes: a molecular dynamics simulation study
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Cross-domain binding of anti-fibrillation peptide TNGQ to islet amyloid polypeptide provides cytoprotective effects in giant unilamellar vesicles and pancreatic β-cells
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Dopamine synthesis and transport: current and novel therapeutics for parkinsonisms
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Gαs and Gαq/11 protein coupling bias of two AVPR2 mutants (R68W and V162A) that cause nephrogenic diabetes insipidus
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Pharmacoperone drugs: targeting misfolded proteins causing lysosomal storage-, ion channels-, and G protein-coupled receptors-associated conformational disorders
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Protein quality control in the secretory pathway
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Cell-trafficking impairment in disease-associated LPA6 missense mutants and a potential pharmacoperone therapy for autosomal recessive woolly hair/hypotrichosis
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Insight into the specificity and severity of pathogenic mechanisms associated with missense mutations through experimental and structural perturbation analyses
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TF-MAPS: fast high-resolution functional and allosteric mapping of DNA-binding proteins
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The Role of the Rhomboid Superfamily in ER Protein Quality Control: From Mechanisms and Functions to Diseases
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Folding, misfolding, and regulation of intracellular traffic of G protein‐coupled receptors involved in the hypothalamic–pituitary–gonadal axis
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Protein aggregation: A detrimental symptom or an adaptation mechanism?
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Regulation of Cannabinoid and Opioid Receptor Levels by Endogenous and Pharmacological Chaperones
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Mutations in G Protein–Coupled Receptors: Mechanisms, Pathophysiology and Potential Therapeutic Approaches
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SRD005825 Acts as a Pharmacologic Chaperone of Opsin and Promotes Survival of Photoreceptors in an Animal Model of Autosomal Dominant Retinitis Pigmentosa
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Performance of Web tools for predicting changes in protein stability caused by mutations
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A case report of classic galactosemia with a GALT gene variant and a literature review
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Demonstrating aspects of multiscale modeling by studying the permeation pathway of the human ZnT2 zinc transporter
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Comparison of the structure of Aβ(1-40) amyloid with the one in complex with polyphenol ε-viniferin glucoside (EVG)
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The melanocortin pathway and control of appetite-progress and therapeutic implications
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Can tolvaptan usage cause cytotoxicity? An in vitro study
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Chaperones as Potential Pharmacological Targets for Treating Protein Aggregation Illness
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Structure of beta-glucosidase A from Paenibacillus polymyxa complexed with a monovalent inhibitor
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ER exit pathways and the control of proteostasis: Crucial role of the UPR, COPII, and ER-phagy in the secretory pathway
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Targeting trafficking as a therapeutic avenue for misfolded GPCRs leading to endocrine diseases
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Defective Gating and Proteostasis of Human ClC-1 Chloride Channel: Molecular Pathophysiology of Myotonia Congenita
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Stabilizing effects of geraniol on native and pathogenic M39R rhodopsin variants
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Osmolytes: A Possible Therapeutic Molecule for Ameliorating the Neurodegeneration Caused by Protein Misfolding and Aggregation
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Ligands for Melanocortin Receptors: Beyond Melanocyte-Stimulating Hormones and Adrenocorticotropin
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Chronic Mexiletine Administration Increases Sodium Current in Non-Diseased Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes
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Hyperosmotic Stress Induces the Expression of Organic Osmolyte Transporters in Porcine Intestinal Cells and Betaine Exerts a Protective Effect on the Barrier Function
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Putative Roles and Therapeutic Potential of the Chaperone System in Amyotrophic Lateral Sclerosis and Multiple Sclerosis
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Exploring Cellular Stress Response and Chaperones
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The Spectrum of Mutations of Homocystinuria in the MENA Region
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Pharmacological Chaperones: A Therapeutic Approach for Diseases Caused by Destabilizing Missense Mutations
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Improvement of Mutant Galactose-1-Phosphate Uridylyltransferase (GALT) Activity by FDA-Approved Pharmacochaperones: A Preliminary Study
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Galactosemia: Towards Pharmacological Chaperones
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Nanomaterials for Modulating the Aggregation of β-Amyloid Peptides
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Pharmacological Chaperone Therapy for Pompe Disease
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Protein Misfolding in Pregnancy: Current Insights, Potential Mechanisms, and Implications for the Pathogenesis of Preeclampsia
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Global Landscape of SLC37A4 Variants and Their Potential Amenability to Pharmacological Chaperone Therapy in Glycogen Storage Disease Type Ib
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Protein synthesis modulation as a therapeutic approach for amyotrophic lateral sclerosis and frontotemporal dementia
inferred← citeshttps://doi.org/10.4103/ijnpnd.ijnpnd_241_25openalex-snapshot
Molecular and Technological Advances in the Treatment of Protein Misfolding Disorders
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Role of misfolding in rare enzymatic deficits and use of pharmacological chaperones as therapeutic approach
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913578openalex-snapshot
Additional file 10 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913581openalex-snapshot
Additional file 11 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913584openalex-snapshot
Additional file 12 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913590openalex-snapshot
Additional file 14 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913593openalex-snapshot
Additional file 1 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913596openalex-snapshot
Additional file 2 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913599openalex-snapshot
Additional file 3 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913602openalex-snapshot
Additional file 4 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913605openalex-snapshot
Additional file 5 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913608openalex-snapshot
Additional file 6 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913611openalex-snapshot
Additional file 7 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913614openalex-snapshot
Additional file 8 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.6084/m9.figshare.14913617openalex-snapshot
Additional file 9 of Performance of Web tools for predicting changes in protein stability caused by mutations
inferred← citeshttps://doi.org/10.65329/wjeb.v13.02.002openalex-snapshot
From Definition to Therapy: A Comprehensive Article Review of Galactosemia
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